An Overt Gastrointestinal Bleeding and Splenomegaly without Portal Hypertension in Celiac Disease

A report describes a case of a 13-year-old female child who presented with pain in the abdomen, melena, and anemia of 8-day duration with a history of a similar episode a month ago. Her lab investigations revealed iron-deficiency anemia, normal LFT, coagulation profiles, and Hb electrophoresis. Abdominal ultrasound demonstrated splenomegaly, a normal-sized liver and portal vein, and no varices or ascites. Her esophagogastroduodenoscopy showed scalloping of the duodenal folds and fundic erosion without varices. 

Her distal duodenal biopsy report was consistent with celiac disease fulfilling the Marsh stage 3b criteria. Her IgA anti-tissue transglutaminase was also positive. 

The patient's Contrast-enhanced computed tomography (CECT) and CT angiography of the abdomen and colonoscopy were normal, and the Meckel scan was negative. The patient was instructed to consume a gluten-free diet and was discharged with PPI and iron supplements, with close follow-up. 

The patient remained symptom-free in the follow-up years with no recurrence of bleeding and with regression of splenomegaly and a rise in the hemoglobin level.

Overt gastrointestinal bleeding in celiac disease patients may suggest associated malignancy, ulcerative jejunoileitis, vitamin K deficiency, or portal varices; however, this case did not show any specific cause of overt GI blood loss, and the physicians could not examine the small bowel to get the etiology of the bleeding exactly. Although splenic atrophy is an established extraintestinal manifestation of CD, it may also exists with splenomegaly with no evidence of IPH (absence of varices and ascites) Aforementioned was one such case of CD with splenomegaly without IPH.

Hence, the celiac disease must be considered a differential diagnosis in patients with either occult or overt GI blood loss, and its extraintestinal complications should be remembered.

An Overt Gastrointestinal Bleeding and Splenomegaly Without Portal Hypertension in Celiac Disease. Indian Journal of Pediatrics. 2023. https://www.ijppediatricsindia.in/an-overt-gastrointestinal-bleeding-and-splenomegaly-without-portal-hypertension-in-celiac-disease-2/